Sjögren’s Syndrome: An Update on Pathogenesis, Clinical Insights, and Treatment

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Immunology".

Deadline for manuscript submissions: 25 May 2024 | Viewed by 5532

Special Issue Editor


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Guest Editor
Rheumatology Department, Hospital of the Holy Cross and Saint Paul, Barcelona, Spain
Interests: sjögren's syndrome; arthritis; doppler ultrasound; rheumatoid arthritis; autoantibodies; rheumatic diseases; systemic lupus erythematosus; autoimmune disease; clinical immunology; clinical rheumatology

Special Issue Information

Dear Colleagues,

Sjögren’s syndrome (SjS) is a heterogeneous systemic disease. The abnormal responses to La/SSB and Ro/SSA of both B-cells and T-cells are implicated in the destruction of the epithelium of exocrine glands, whose tissue characteristically shows a peri-epithelial lymphocytic infiltration that can vary from sicca syndrome to systemic disease and lymphoma. Novel diagnostic laboratory techniques and image tools have emerged in recent years, allowing early diagnosis with better organic diagnostic accuracy. Moreover, in addition to commonly used therapies, a large amount of knowledge has emerged for treating SjS with novel biological therapies. The window of opportunity for clinicians and researchers is open to help to prevent organ damage in patients.

We invite you to participate in this Special Issue of JCM on Sjögren’s syndrome (SjS). As you certainly know, SjS is a chronic disorder that causes insufficient moisture production in certain glands of the body, including the saliva-producing and tear-producing glands. Glandular disease is largely common, but extraglandular organ damage can make it challenging for patients and their physicians. Early diagnosis and appropriate treatment are essential for optimal management of SjS.

In this Special Issue titled “Sjögren’s Syndrome: An Update on Pathogenesis, Clinical Insights, and Treatment”, we welcome authors to submit original clinical or experimental studies with emphasis on Sjögren’s Syndrome.

We look forward to receiving your submissions to this Special Issue.

Dr. Hèctor Corominas
Guest Editor

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Keywords

  • sjögren’s syndrome
  • pathogenesis
  • diagnosis
  • clinical features
  • treatment

Published Papers (4 papers)

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Research

11 pages, 915 KiB  
Article
The Croatian Primary Sjögren’s Disease Oral Health Study: Oral Status and Oral Health-Related Quality of Life
by Ana Glavina, Ivona Božić, Katica Parat, Dijana Perković, Dolores Biočina-Lukenda, Dušanka Martinović Kaliterna and Mislav Radić
J. Clin. Med. 2023, 12(14), 4804; https://doi.org/10.3390/jcm12144804 - 21 Jul 2023
Cited by 1 | Viewed by 904
Abstract
To determine salivary flow rate, oral and periodontal status, oral health-related quality of life (OHRQoL), objective and subjective indexes, and serum antibody reactivity in patients with primary Sjögren’s disease (pSD). Thirty-one patients with pSD and 31 control subjects participated in this cross-sectional, single-center [...] Read more.
To determine salivary flow rate, oral and periodontal status, oral health-related quality of life (OHRQoL), objective and subjective indexes, and serum antibody reactivity in patients with primary Sjögren’s disease (pSD). Thirty-one patients with pSD and 31 control subjects participated in this cross-sectional, single-center study. The unstimulated whole salivary flow rate (UWSFR) and stimulated whole salivary flow rate (SWSFR), salivary pH, DMFT index (DMFT = D—decayed, M—missing, F—filled tooth), periodontal pocket depth (PPD), clinical attachment level (CAL), interincisal distance, OHRQoL, objective European League Against Rheumatism (EULAR) SS Disease Activity Index (ESSDAI) and subjective (EULAR SS Patient Reported Index (ESSPRI), 6-items-VAS-SS (Visual Analog Scale), Profile of Fatigue) indexes were analyzed. The patients with pSD had a blood sample taken in the morning between 7 and 10 a.m. for comprehensive laboratory analysis. Patients with pSD had statistically significant lower UWSFR (0.20 vs. 0.90 mL/min) and SWSFR (0.56 vs. 1.64 mL/min) values compared with control subjects (p < 0.001, Mann-Withney U test). Salivary pH value of pSD patients was significantly lower compared with control subjects (6.00 vs. 7.00; p < 0.001, Mann-Whitney U test). The mean DMFT index of patients with pSD compared to control subjects was not statistically significant (23.74 ± 7.28 vs. 20.77 ± 5.73; p = 0.08, t-test). Interincisal distance was significantly decreased in the pSD group compared with control subjects (43.80 ± 0.38 vs. 47.60 ± 0.50; p = 0.003, t-test). The prevalence of periodontitis was similar in patients with pSD and control subjects (83.9% vs. 77.4%; p = 0.35, λ2 test). The mean Oral Health Impact Profile (OHIP-49) total score was statistically significantly higher in pSD patients compared with control subjects (32.00 vs. 8.00; p < 0.001, Mann–Whitney U test). Patients with pSD have decreased salivary flow and salivary pH, poor oral health, decreased interincisal distance, high prevalence of periodontitis, and worse OHRQoL. These findings highlight the need for a multidisciplinary approach to the management of patients with pSD that includes physical and psychological aspects of the disease. Full article
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10 pages, 252 KiB  
Article
Increased Risk of Developing Systemic Lupus Erythematosus and Rheumatoid Arthritis in Patients with Primary Sjögren’s Syndrome—A Secondary Cohort Analysis of Nationwide, Population-Based Claims Data
by Malcolm Koo, Chia-Wen Hsu and Ming-Chi Lu
J. Clin. Med. 2023, 12(12), 4157; https://doi.org/10.3390/jcm12124157 - 20 Jun 2023
Cited by 1 | Viewed by 971
Abstract
Background: This retrospective cohort study aimed to examine the risk of developing systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) in patients with primary Sjögren’s syndrome (pSS) compared to controls using data from a nationwide health claims database. Methods: Four distinct cohorts of [...] Read more.
Background: This retrospective cohort study aimed to examine the risk of developing systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) in patients with primary Sjögren’s syndrome (pSS) compared to controls using data from a nationwide health claims database. Methods: Four distinct cohorts of patients with newly diagnosed pSS were established using Taiwan’s National Health Insurance Research Database. Cohorts I and II were created to evaluate the risk of developing SLE and RA, respectively. Cohorts III and IV were assembled similarly to Cohorts I and II but employed a stricter definition, based on catastrophic illness certificate (CIC) status, for identifying patients with pSS. Comparison cohorts of patients without pSS were formed by frequency matching for sex, 5-year age interval, and index year. Incident rate ratios (IRR) for SLE or RA development were determined using Poisson regression models. Results: Patients with pSS, selected from just outpatient visits or with additional CIC status showed a significantly higher risk of developing SLE or RA compared with the controls. When stratified by age group or sex, the risk of developing SLE was notably higher in the young age group (adjusted IRR 47.24, p = 0.002) and women (adjusted IRR 7.63, p = 0.003) among patients with pSS. In addition, both men and women with pSS, irrespective of age, showed a significantly elevated risk of developing RA. Conclusions: Patients with pSS exhibited an elevated risk of developing SLE and RA. Rheumatologists should carefully monitor patients with pSS for potential SLE and RA development. Full article
16 pages, 2155 KiB  
Article
Comparative Analysis of Hematological and Immunological Parameters in Patients with Primary Sjögren’s Syndrome and Peripheral Neuropathy
by Ancuta Mihai, Diana Maria Chitimus, Ciprian Jurcut, Florin Cristian Blajut, Daniela Opris-Belinski, Constantin Caruntu, Ruxandra Ionescu and Ana Caruntu
J. Clin. Med. 2023, 12(11), 3672; https://doi.org/10.3390/jcm12113672 - 25 May 2023
Cited by 2 | Viewed by 1426
Abstract
Background: Primary Sjögren syndrome (pSS) is a multisystem disorder of autoimmune etiology, frequently involving peripheral nerves. Early detection of peripheral neuropathy (PN) manifestations might improve prognosis and disease control. The purpose of the study was to evaluate the predictive potential of hematological and [...] Read more.
Background: Primary Sjögren syndrome (pSS) is a multisystem disorder of autoimmune etiology, frequently involving peripheral nerves. Early detection of peripheral neuropathy (PN) manifestations might improve prognosis and disease control. The purpose of the study was to evaluate the predictive potential of hematological and immunological parameters associated with PN development in pSS patients. Methods: This single-center retrospective study included patients with pSS who were divided into two groups, according to the occurrence of neurological manifestations throughout the follow-up period. Results: From the total of 121 pSS patients included in the study, 31 (25.61%) developed neurological manifestations (PN+ group) during the follow-up period. At the moment of pSS diagnosis, 80.64% of PN+ patients exhibited increased disease activity, with ESSDAI scores above 14 (p = 0.001), and significantly higher values for VASp score (p = 0.001), with a mean value of 4.90 ± 2.45, compared to 1.27 ± 1.32 in the PN- group. The hematological assessment at the moment of pSS diagnosis revealed that neutrophils and neutrophil-to-lymphocyte ratio (NLR) were significantly higher in the PN+ group (p = 0.001), while lymphocytes, monocytes and monocyte-to-lymphocyte ratio (MLR) were significantly lower (p = 0.025, p = 0.13 and p = 0.003, respectively). Immuno-inflammatory parameters—gammaglobulins, complement fractions C3, C4, total proteins and vitamin D were significantly lower in the PN+ patients’ group. In multivariate analysis, the independent predictive character for PN development in pSS patients was confirmed for NLR (95% CI 0.033 to 0.263, p = 0.012), MLR (95% CI −1.289 to −0.194, p = 0.008), gammaglobulins (95% CI −0.426 to −0.088, p < 0.003), complement fraction C4 (95% CI −0.018 to −0.001, p < 0.030) and vitamin D (95% CI −0.017 to −0.003, p < 0.009). Conclusions: Readily available and frequently used hematological and immunological markers, such as NLR, MLR, gammaglobulins, C4 and vitamin D could be helpful in predicting the neurological involvement in pSS patients. These biological parameters might become useful tools for clinicians to monitor disease progression and identify potentially severe extraglandular manifestations in pSS patients. Full article
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11 pages, 965 KiB  
Article
Diagnostic Evaluation Using Salivary Gland Ultrasonography in Primary Sjögren’s Syndrome
by Yen-Fu Chen, Ao-Ho Hsieh, Yao-Fan Fang and Chang-Fu Kuo
J. Clin. Med. 2023, 12(6), 2428; https://doi.org/10.3390/jcm12062428 - 22 Mar 2023
Cited by 1 | Viewed by 1830
Abstract
The purpose of this study is to investigate the clinical manifestations in patients with early primary Sjögren’s syndrome (pSS) based on the severity score found by salivary gland ultrasonography. Thirty-five newly diagnosed patients with early pSS were enrolled and divided into mild (score [...] Read more.
The purpose of this study is to investigate the clinical manifestations in patients with early primary Sjögren’s syndrome (pSS) based on the severity score found by salivary gland ultrasonography. Thirty-five newly diagnosed patients with early pSS were enrolled and divided into mild (score 0–1) and severe (score 2–3) groups according to the salivary gland ultrasonography grade (SGUS) scores at baseline. Clinical evaluation, ESSPRI and ESSDAI index values, sicca symptoms of the mouth, salivary capacity, and serum autoantibodies and cytokines were investigated. The mean age of pSS patients at diagnosis was 49.9 ± 11.9 years, and the mean duration of sicca symptoms was 0.58 years. ESSPRI (EULAR Sjögren’s syndrome patient report index) and ESSDAI (EULAR Sjögren’s syndrome disease index) scores were 15.97 and 4.77, respectively. Clinical manifestations, including the low production of saliva and autoantibody production, such as antinuclear antibodies, rheumatoid factor, and anti-SSA antibody, were found. A higher prevalence of rheumatoid factor (p = 0.0365) and antinuclear antibody (p = 0.0063) and a higher elevation of total IgG (p = 0.0365) were found in the severe group than in the mild group. In addition, the elevated titer of IL-25 was detected in the severe group than in the mild group. This observation indicated that salivary gland ultrasonography grade (SGUS) scans may help physicians diagnose pSS and the elevated titer of IL-25 in patients may be implicated in the pathogenesis of pSS. Full article
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